Mouse Genome Informatics
cn
    Tg(CAG-cat,-Ptpn11*Q97R)1Rbns/0
Tg(Tek-cre)12Flv/0

involves: C3H * C57BL/6 * FVB/N
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
mortality/aging
• fewer than expected mice are present at E13.5 and no mice are present after birth

cardiovascular system
• at E13.5, valve primordial has not yet undergone differentiation and extracellular remodeling as in wild-type mice
• cushion volume is increased up to 85% compared to in wild-type mice
• outflow tract cushion volume is only increased by 39% compared to in wild-type mice
• in some mice
• proliferation of endothelial, mesenchymal, and cardiomyocyte cells is increased compared to in wild-type
• proliferation of cells in the atrioventricular canal cushion endothelial and mesenchymal cell proliferation is increased while apoptosis is decreased compared to in wild-type mice
• however, proliferation of cardiomyocytes in the atrioventricular canal cushion is normal
• at E13.5
• some mice exhibit ventricular noncompaction

embryogenesis

growth/size
• at E13.5

homeostasis/metabolism
• at E13.5, mice exhibit nuchal and back edema
• at E13.5

liver/biliary system
• at E13.5

muscle
• some mice exhibit ventricular noncompaction

integument
• at E13.5

Mouse Models of Human Disease
OMIM IDRef(s)
Noonan Syndrome 1; NS1 163950 J:142212